Description of eyelid malformation in Fraser's syndrome

Goal : Fraser's syndrome is a rare polymalformation involving the eyes, wit h cryptophthalmos. In its complete form, cryptophthalmos is a serious malfo mation with absence of eyelids and palpebral fissure. A review of ophthalmo logical signs is presented. Case report: A case of Fraser's syndrome is presented. Ophthalmological and somatic examination as well as evolution are described. Results:Incomplete cryptophthalmos with symblepharon and bilateral palpebra l coloboma are present. Surgical treatment with palpebral reconstruction al lowed preservation of visual acuity in one eye. Discussion : Ophthalmological signs are present in 93% of cases. Eyelid inv olvement such as symblepharon, and partial or complete coloboma are relativ ely frequent, whereas cryptophthalmos in its complete form are much more ra re. Conclusion : Treatment involves palpebal reconstructive surgery in order to preserve visual function.